{
  "abstract": "Introduction Interstitial lung disease (ILD) is a leading cause of mortality in systemic sclerosis (SSc). Half present with mild disease, of whom 25% progress over two years. Our aim was to explore demographic, clinical, and serological predictors of progression in mild SSc-ILD.Material and Methods We conducted an international retrospective cohort study using data from the Canadian Scleroderma Research Group (CSRG), Australian Scleroderma Cohort Study (ASCS), Centre hospitalier de l’Université de Montréal (CHUM), and Stanford University SSc cohorts. Patients were included if they had ILD, forced vital capacity (FVC) >= 80% predicted, and available follow-up pulmonary function test data. ILD was diagnosed on HRCT or, if unavailable, then by chest X-ray or on physical exam with the presence of typical Velcro-like crackles, based on a published algorithm. ILD progression was defined as a relative decrease of at least 10% in FVC, or a relative decrease of 5 to 10% in FVC with a relative decline of at least 15% in DLCO. Multivariable Cox regression analyses were used to analyze the association between each predictor variable and time to ILD progression, adjusting for age, sex, ethnicity, smoking, and disease duration from first non-Raynaud symptoms.Results A total of 591 patients were analyzed, including 280 CSRG, 232 ASCS, 56 CHUM and 23 Stanford patients. Mean age was 58.8 ± 11.8 years, 84% were female, 87% were White, 44% had diffuse SSc, and median disease duration was 7.4 [2.9, 15.9] years at time of mild ILD diagnosis. Of these, 304 patients progressed over a mean follow-up of 3.6 years. Factors associated with a shorter time to ILD progression included age (HR: 1.01, 95% CI: 1.00, 1.02), non-White ethnicity (HR: 1.41, 95% CI: 1.02, 1.95), diffuse subtype (HR: 1.29, 95% CI: 1.01, 1.64), higher modified Rodnan skin score (mRSS, HR: 1.07, 95% CI: 1.00, 1.13 for every 5-unit increase), and lower % predicted DLCO (HR: 0.96, 95% CI: 0.93, 1.00 for every 5-unit increase). SSc-specific antibodies were not associated with progression.Conclusions In this international cohort, we identified risk factors associated with a shorter time to ILD progression in mild forms of SSc-ILD. These features warrant closer monitoring and earlier treatment in this population and can inform enrichment strategies for interventional trials.Abstract P.108 Table 1Demographic, clinical and serological characteristics associated with risk of mild SSc-ILD progression, in unadjusted and adjusted models",
  "authors": [
    {
      "affiliations": [
        "University of Montreal - Division of Rheumatology, Montreal, Canada"
      ],
      "name": "Sabrina Hoa"
    },
    {
      "affiliations": [
        "University of Montreal - Division of Rheumatology, Montreal, Canada"
      ],
      "name": "Danick Goulet"
    },
    {
      "affiliations": [
        "Stanford University, Division of Immunology and Rheumatology, Stanford, USA"
      ],
      "name": "Yumeko Kawano"
    },
    {
      "affiliations": [
        "Royal Adelaide Hospital, University of Adelaide, Division of Rheumatology, Adelaide, Australia"
      ],
      "name": "Susanna Proudman"
    },
    {
      "affiliations": [
        "St. Vincent’s Hospital Melbourne, Fitzroy, Division of Rheumatology, Victoria, Australia"
      ],
      "name": "Wendy Stevens"
    },
    {
      "affiliations": [
        "Stanford University, Division of Immunology and Rheumatology, Stanford, USA"
      ],
      "name": "Lorinda Chung"
    },
    {
      "affiliations": [
        "University of Montreal - Division of Rheumatology, Montreal, Canada"
      ],
      "name": "Amine Mohamed Maimoune"
    },
    {
      "affiliations": [
        "University of Montreal - Division of Rheumatology, Montreal, Canada"
      ],
      "name": "Raphael Hurtubise"
    },
    {
      "affiliations": [
        "University of Calgary, Cumming School of Medicine, Division of Rheumatology, Calgary, Canada"
      ],
      "name": "Maggie Larché"
    },
    {
      "affiliations": [
        "University of Calgary, Cumming School of Medicine, Division of Rheumatology, Calgary, Canada"
      ],
      "name": "May Choi"
    },
    {
      "affiliations": [
        "University of Alberta, Division of Rheumatology, Edmonton, Canada"
      ],
      "name": "Mohammed Osman"
    },
    {
      "affiliations": [
        "University of Western Ontario, Division of Rheumatology, London, Canada"
      ],
      "name": "Janet Pope"
    },
    {
      "affiliations": [
        "Southlake Regional Health Centre, University of Toronto, Newmarket, Canada"
      ],
      "name": "Carter Thorne"
    },
    {
      "affiliations": [
        "McGill University, Division of Rheumatology, Montreal, Canada"
      ],
      "name": "Murray Baron"
    },
    {
      "affiliations": [
        "University of Sydney, Division of Rheumatology at Royal Prince Alfred Hospital and School of Public Health, Sydney, Australia"
      ],
      "name": "Mandana Nikpour"
    },
    {
      "affiliations": [
        "McGill University, Division of Rheumatology, Montreal, Canada"
      ],
      "name": "Marie Hudson"
    },
    {
      "affiliations": [
        "Canadian Scleroderma Research Group, Canada"
      ],
      "name": "Canadian Scleroderma Research"
    },
    {
      "affiliations": [
        "Australian Scleroderma Interest Group, Australia"
      ],
      "name": "Australian Scleroderma Interest"
    }
  ],
  "title": "P.108 Predicting progression of mild interstitial lung disease in systemic sclerosis: an international retrospective cohort study",
  "uid": "4470d074-82f7-5f1c-8372-21c52cf223c0"
}